Lambert?Eaton myasthenic syndrome (LEMS) is an autoimmune disease of the neuromuscular junction and approximately 60% of LEMS patients have a tumor, mostly small cell lung cancer, as a paraneoplastic neurological syndrome. LEMS patients develop a unique set of clinical characteristics, which include proximal muscle weakness, depressed tendon reflexes with post-tetanic potentiation and autonomic symptoms. Interestingly, slightly <10% of LEMS patients have cerebellar ataxia (LEMS with paraneoplastic cerebellar degeneration). Considering its pathomechanism, LEMS is a presynaptic disorder of neuromuscular transmission in which quantal release of acetylcholine is impaired by autoantibodies for P/Q-type voltage-gated calcium channels at active zones, although an animal model by immunizing purified P/Q-type voltage-gated calcium channels has not yet been successful.
雑誌名
Clinical and Experimental Neuroimmunology
巻
7
号
3
ページ
238 - 245
発行年
2016-08
出版者
日本神経免疫学会
出版者別言語
Japanese Society for Neuroimmunology
ISSN
17591961
DOI
10.1111/cen3.12326
権利
c 2016 Japanese Society for Neuroimmunology
著者版フラグ
publisher
引用
Clinical and Experimental Neuroimmunology, 7(3), pp.238-245; 2016
Lambert?Eaton myasthenic syndrome: Clinical review
CEN7_238.pdf
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